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Medical Author:

Robert Ferry Jr., MD

Robert Ferry Jr., MD

Robert Ferry Jr., MD, is a U.S. board-certified Pediatric Endocrinologist. After taking his baccalaureate degree from Yale College, receiving his doctoral degree and residency training in pediatrics at University of Texas Health Science Center at San Antonio (UTHSCSA), he completed fellowship training in pediatric endocrinology at The Children's Hospital of Philadelphia.

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Medical Editor:

Melissa Conrad Stöppler, MD, Chief Medical Editor

Melissa Conrad Stöppler, MD, Chief Medical Editor

Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

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Pineal region tumors arise in or near the pineal gland, which is a small midline structure located deep in the midbrain area, near many vital structures. The pineal gland is located next to the aqueduct of Sylvius, which serves as a passage allowing cerebrospinal fluid (CSF) to leave the center of the brain where it is first produced. Pineal tumors often compress this aqueduct, causing a build up of pressure of CSF in the brain (called hydrocephalus). Blockage of the flow of this fluid can cause some of the common presenting symptoms of these tumors, which include:

• headache,
  
  
• nausea and vomiting,
  
  
• seizures,
  
  
• memory disturbances, and
  
  
• visual changes.

The intracranial pressure may even increase to life-threatening levels, demanding urgent treatment.

Hydrocephalus can be treated by placement of a ventriculo-peritoneal shunt (VP shunt). The VP shunt is a long tube placed within one of the CSF-containing spaces of the brain, then passed under the skin to the abdominal cavity to provide a pathway for CSF drainage and absorption in the abdomen.

Alternatively, the hydrocephalus can be controlled by a procedure known as a stereotactic third ventriculostomy. Third ventriculostomy creates a tiny opening in the bottom of the brain using a small endoscope to allow the CSF to escape. This procedure is usually performed under local anesthesia (without the need for general anesthesia).

Pineal region tumors may also cause visual changes as a result of involvement of the nearby tectal region which has a primary role in controlling eye movements. These changes may include:

• inability to focus on objects,
  
  
• double vision, and
  
  
• impairment of eye movements.

These problems may improve or resolve with treatment of the tumor. Certain germ cell tumors may secrete hormones which cause endocrinologic disturbances, such as early onset of puberty in children.

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